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Unusual presentation of a case of Sjogren's syndrome with neurological and ocular manifestation
Contact Lens & Anterior Eye 35 (2012) 85–88
Contents lists available at SciVerse ScienceDirect
Contact Lens & Anterior Eye journal homepage: www.elsevier.com/locate/clae
Case report
Unusual presentation of a case of Sjogren’s syndrome with neurological and ocular manifestation Naina R. Bamrolia ∗ , Ritu Arora, Usha Yadava Guru Nanak Eye Centre, Maulana Azad Medical College, New Delhi 110002, India
a r t i c l e
i n f o
Keywords: Sjogren’s syndrome Pseudomembraneous conjunctivitis Conjunctival necrosis Cyclosporine
a b s t r a c t Sjogren’s syndrome is an autoimmune disease that commonly presents to the ophthalmologist as a dry eye disease. We report an unusual presentation of a case of Sjogren’s syndrome. A 33-year-old man presented with lid swelling, pseudomembraneous conjunctivitis with central corneal epithelial defect and history of limb weakness for past 2 years. There was progressive enlargement of the epithelial defect and conjunctival-scleral necrosis developed during follow-up. Evaluation for underlying connective tissue disorder was positive for SS-B/La antibody and, the rheumatoid factor, anti nuclear antibody, anti neutrophil cytoplasmic antibody titres were negative. Patient was diagnosed as a case of Primary Sjogren’s syndrome. Resolution of the conjunctival-scleral necrosis occurred following treatment with combination of cyclosporine and prednisolone. Paramedian tarsorrhaphy was performed to promote healing of the corneal epithelium. The corneal epithelium healed, conjunctival-scleral necrosis resolved completely and the visual acuity improved to 6/36 in the right eye after 3 months of immunosuppressive therapy. The immunosuppressive therapy was discontinued after completion of 6 months of treatment. After 6 months follow-up of stopping immunosuppressive therapy, the ocular and systemic condition is stable. © 2011 British Contact Lens Association. Published by Elsevier Ltd. All rights reserved.
1. Introduction Primary Sjogren’s syndrome, previously diagnosed or undiagnosed often present to the ophthalmologist with typical signs and symptoms of dry eye disease [1]. Severe ocular surface inflammation in the form of pseudomembraneous conjunctivitis and conjunctival-scleral necrosis in cases of Sjogren’s syndrome has not been reported previously. We report a case of Sjogren’s syndrome where pseudomembraneous conjunctivitis and conjunctival-scleral necrosis occurred as a manifestation of severe ocular surface inflammation and which resolved after combination therapy with oral prednisolone and cyclosporine. 2. Case report A 33-year-old man presented with complaint of diminution of vision in his right eye which was associated with severe swelling and drooping of the upper lid (Fig. 1A) for 5 days. Past history revealed that he had episodic limb weakness for the last 2 years. Although his limb weakness resolved completely within 5 days of the recent episode, he noticed redness and swelling of the upper
∗ Corresponding author at: 123B Gautam Nagar, New Delhi 110049, India. Tel.: +91 9868918469. E-mail address: [email protected] (N.R. Bamrolia).
lid of right eye associated with diminution of vision for which he presented to us. On physical examination, there was no neurological deficit but he had dryness of mouth. On ocular examination, his visual acuity was counting fingers close to face in the right eye, and 6/6 in the left. There was mechanical ptosis due to swelling of the upper lid of the right eye. Slit lamp evaluation of the right eye showed a 3 mm × 4 mm size epithelial defect in the centre of cornea without any infiltrate (Fig. 2A), congestion of the bulbar conjunctiva (Fig. 2B) and a thick pseudomembrane on the upper palpebral conjunctiva which was adherent to adjacent bulbar conjunctiva. It was difficult to separate the pseudomembrane from the underlying conjunctiva (Fig. 1B). There was absence of anterior chamber inflammation and hypopyon, and the fundus was not visible in the right eye. The ocular examination was normal in the left eye. He was prescribed topical carboxy methyl cellulose 0.5% lubricant drops and ointment for night application and referred to a neurologist for further evaluation of his systemic disease. Haematological, immunological and radiological investigations were performed. Haematological investigations were normal except for ESR which was raised. (haemogram – 12.5 gm/dl, differential count: P – 64%, L – 32%, E – 0, M – 0, B – 0, platelet count – 2 lakh/mm3 , ESR – 42 mm/1st hour). Blood urea, creatinine, bilirubin, aminotransferases, alkaline phosphatase, electrolytes, lipid profile, fasting and postprandial blood sugar and 24 h urine vanillylmandelic acid were normal. On immunological tests for connective tissue disorder, the SS-B/La antibody titre was raised (10.33 U/ml) but the SS-A/Ro was
1367-0484/$ – see front matter © 2011 British Contact Lens Association. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.clae.2011.10.002
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N.R. Bamrolia et al. / Contact Lens & Anterior Eye 35 (2012) 85–88
Fig. 1. (A) Clinical photograph of the patient at presentation revealing swelling and inflammation of upper lid of the right eye. (B) Clinical photograph of the upper lid of right eye of the patient revealing thick pseudomembrane on the palpebral conjunctiva. (C) Resolution of swelling and inflammation of upper lid following addition of cyclosporine. (D) Resolution of pseudomembrane on palpebral conjunctiva following addition of cyclosporine.
normal (0.8 U/mL) while the rheumatoid factor (<10 IU/mL; normal <10 IU/mL), anti nuclear antibody, anti DsDNA(4.0 IU/mL; normal <20 IU/mL), anti neutrophil cytoplasmic antibody titres (pANCA and cANCA) were negative. Radiological investigation including chest radiograph and contrast enhanced computed tomography of the abdomen were normal. As the patient did not have the typical features of other connective tissue disorders and fulfilled four of the six criteria of the revised international classification criteria for Sjögren’s syndrome (presence of signs and symptoms of dry eye and dry mouth along with positivity of highly specific marker autoantibodies-SSB/La), a diagnosis of primary Sjogren’s syndrome with intermitant pure motor quadriparesis was made. Subsequently, oral prednisolone 60 mg once daily was started by the neurologist and he was instructed to come for biweekly follow up visits. At 4 weeks following his initial presentation, his ocular condition had progressively deteriorated. Slit lamp evaluation revealed a large corneal epithelial defect of size 7 mm × 8 mm, with stromal haze. The anterior chamber structures could not be visualized and large patch of conjunctival-scleral necrosis of 14 mm × 7 mm in size, extending 1 mm from the limbus on the superotemporal bulbar conjunctiva was noted (Fig. 2C and D). In view of the progressive worsening of the ocular surface, oral cyclosporine 50 mg thrice daily was added, after consultation with the neurologist. After 1 week of oral cyclosporine therapy, although the conjunctival-scleral necrosis and pseudomembrane started showing signs of resolution, the corneal epithelial defect persisted (Figs. 1C and D, 2E and F). In view of presence of the non-healing corneal epithelial defect and progressive onset of cicatrial entropion, a paramedian tarsorrhaphy was performed with minimal scrapping of the lid margin due to the risk of aggravating the inflammation and necrotic process further. The tissue scrapings were insufficient for histopathological evaluation. After 3 months of immunosuppressive therapy, the visual acuity improved to 6/36, the conjunctival-scleral necrosis had resolved and the corneal epithelium had healed (Fig. 2G and H). Dose of prednisolone was tapered to 10 mg once daily after 1 month of addition of cyclosporine. Immunosuppressive therapy was discontinued after completion of 6 months of treatment. The ocular and systemic condition is presently clinically stable after stopping of immunosuppressive therapy for 6 months. 3. Discussion Sjogren’s syndrome is a multisystem disease that often presents as a dry eye and dry mouth complex due to involvement of
the lacrimal and salivary gland [1]. Up to one third of patients of Sjogren’s syndrome present with extraglandular manifestation involving lung, liver, kidney, blood vessels, and the nervous system [2]. In some patients neurological manifestations may precede ophthalmic manifestation [3]. Due to variability in the presentation of the disease, the diagnosis is often delayed. Similarly, in our case the diagnosis was delayed by 3 years. Our patient presented with neurological manifestation as intermitant quadriparesis preceding an atypical ocular presentation in the form of lid swelling causing mechanical ptosis associated with signs of severe ocular surface inflammation including pseudomembraneous conjunctivitis and conjunctival-scleral necrosis. The diagnosis of Sjogren’s syndrome was made after excluding other connective tissue disorders (wegner’s granulomatosis, systemic lupus erythematosis, rheumatoid arthritis) which may also be associated with ocular manifestations. Wegner’s granulomatosis which also presents as necrotising keratoscleritis was clinically excluded in our patient due to the absence of pulmonary, renal and sinus involvement, along with absence of cANCA and pANCA positivity [4]. Moreover this disease is very rare in India [5]. Conjunctivalscleral necrosis has been described in systemic vasculitis such as Wegner’s granulomatosis, however to the best of our knowledge pseudomembraneous conjunctivitis with conjunctival-scleral necrosis as the presenting feature of Sjogren’s syndrome has not been described previously. The extraglandular features of Sjogren’s syndrome are often managed with systemic glucocorticoids and immunosuppressive drugs [6]. Due to progression of conjunctival-scleral necrosis despite initial treatment with oral prednisolone alone, oral cyclosporine was added. This combination therapy along with tarsorrhaphy helped us to manage the case satisfactorily. Cyclosporine when combined with corticosteroid has been reported to have better therapeutic efficacy as well as exert steroid sparing effect in various ocular and non-ocular conditions [7–9]. Interestingly, the beneficial effect of the combination of oral cyclosporine and prednisolone for treatment of ocular complications in Sjogren’s syndrome, has been reported only in few cases [10]. Thus patients presenting with unexplained pseudomembraneous conjunctivitis, conjunctival-scleral necrosis and neurological symptoms, should be evaluated with a high index of suspicion for Sjogren’s syndrome with or without systemic vasculitis. Such patients presenting with severe forms of ocular surface inflammation, may benefit from the combination of oral prednisolone and cyclosporine. Nevertheless, the important role of tarsorrhaphy in
N.R. Bamrolia et al. / Contact Lens & Anterior Eye 35 (2012) 85–88
87
Fig. 2. Composite sequential photographs of the right eye of the patient. (A and B) At presentation: (A) A 3 mm × 5 mm corneal epithelial defect (B). Absence of necrosis of bulbar conjunctiva. (C and D) At 4 weeks following presentation: (C) A large corneal epithelial defect of size 7 mm × 8 mm, with stromal haze and non visualization of the anterior chamber structures. (D) A large patch of conjunctival-scleral necrosis 14 mm × 7 mm dimension, 1 mm from the limbus on the superotemporal bulbar conjunctiva was noted. (E and F) After 1 week of oral immunosuppressive therapy: (E) The corneal epithelial defect persisted. (F) The conjunctival-scleral necrosis had resolved markedly. (G and H) At 12 weeks following presentation: (G) Corneal epithelium had healed. (H) Conjunctival-scleral necrosis had resolved completely.
maintaining the tear film and thus facilitating healing of the corneal epithelium should also not be ignored. Conflict of interest None. References [1] Ramos-Casals M, Tzioufas AG, Stone JH, Sisó A, Bosch X. Treatment of primary Sjögren syndrome: a systematic review. JAMA 2010;304(July (4)):452–60.
[2] Shan SJ, Wu EI, Akpek EK. Sterile corneal melt after descemet stripping endothelial keratoplasty in patients with previously undiagnosed Sjogren syndrome. Arch Ophthalmol 2009;127(February (2)):219–20. [3] Alhomoud IA, Bohlega SA, Alkawi MZ, Alsemari AM, Omer SM, Alsenani FM. Primary Sjogren’s syndrome with central nervous system involvement. Saudi Med J 2009;30(August (8)):1067–72. [4] Chua J, Lim L. Systemic Wegener’s granulomatosis with severe orbito-ocular involvement. Singapore Med J 2008;49(October (10)):e259–62. [5] Biswas J, Babu K, Gopal L, Krishnakumar S, Suresh S, Ramakrishnan S. Ocular manifestations of Wegener’s granulomatosis. Analysis of nine cases. Indian J Ophthalmol 2003;51(September (3)):217–23. [6] Wakamatsu TH, Sato EA, Matsumoto Y, Ibrahim OM, Dogru M, Kaido M, Ishida R, Tsubota K. Conjunctival in vivo confocal scanning laser microscopy in patients with Sjögren syndrome. Invest Ophthalmol Vis Sci 2010;51(January (1)):144–50 [Epub 2009 August 20].
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N.R. Bamrolia et al. / Contact Lens & Anterior Eye 35 (2012) 85–88
[7] Panaccione R, Rutgeerts P, Sandborn WJ, Feagan B, Schreiber S, Ghosh S. Review article: treatment algorithms to maximize remission and minimize corticosteroid dependence in patients with inflammatory bowel disease. Aliment Pharmacol Ther 2008;28(September (6)):674–88. [8] Lam DS, Wong AK, Tham CC, Leung AT. The use of combined intravenous pulse methylprednisolone and oral cyclosporin A in the treatment of corneal graft rejection: a preliminary study. Eye (Lond) 1998;12(4):615–8.
[9] Niaudet P, Fuchshuber A, Gagnadoux MF, Habib R, Broyer M. Cyclosporine in the therapy of steroid-resistant idiopathic nephrotic syndrome. Kidney Int Suppl 1997;58:S85–90. [10] Cordero-Coma M, Anzaar F, Sobrin L, Foster CS. Systemic immunomodulatory therapy in severe dry eye secondary to inflammation. Ocul Immunol Inflamm 2007;15(March–April (2)):99–104.
Contents lists available at SciVerse ScienceDirect
Contact Lens & Anterior Eye journal homepage: www.elsevier.com/locate/clae
Case report
Unusual presentation of a case of Sjogren’s syndrome with neurological and ocular manifestation Naina R. Bamrolia ∗ , Ritu Arora, Usha Yadava Guru Nanak Eye Centre, Maulana Azad Medical College, New Delhi 110002, India
a r t i c l e
i n f o
Keywords: Sjogren’s syndrome Pseudomembraneous conjunctivitis Conjunctival necrosis Cyclosporine
a b s t r a c t Sjogren’s syndrome is an autoimmune disease that commonly presents to the ophthalmologist as a dry eye disease. We report an unusual presentation of a case of Sjogren’s syndrome. A 33-year-old man presented with lid swelling, pseudomembraneous conjunctivitis with central corneal epithelial defect and history of limb weakness for past 2 years. There was progressive enlargement of the epithelial defect and conjunctival-scleral necrosis developed during follow-up. Evaluation for underlying connective tissue disorder was positive for SS-B/La antibody and, the rheumatoid factor, anti nuclear antibody, anti neutrophil cytoplasmic antibody titres were negative. Patient was diagnosed as a case of Primary Sjogren’s syndrome. Resolution of the conjunctival-scleral necrosis occurred following treatment with combination of cyclosporine and prednisolone. Paramedian tarsorrhaphy was performed to promote healing of the corneal epithelium. The corneal epithelium healed, conjunctival-scleral necrosis resolved completely and the visual acuity improved to 6/36 in the right eye after 3 months of immunosuppressive therapy. The immunosuppressive therapy was discontinued after completion of 6 months of treatment. After 6 months follow-up of stopping immunosuppressive therapy, the ocular and systemic condition is stable. © 2011 British Contact Lens Association. Published by Elsevier Ltd. All rights reserved.
1. Introduction Primary Sjogren’s syndrome, previously diagnosed or undiagnosed often present to the ophthalmologist with typical signs and symptoms of dry eye disease [1]. Severe ocular surface inflammation in the form of pseudomembraneous conjunctivitis and conjunctival-scleral necrosis in cases of Sjogren’s syndrome has not been reported previously. We report a case of Sjogren’s syndrome where pseudomembraneous conjunctivitis and conjunctival-scleral necrosis occurred as a manifestation of severe ocular surface inflammation and which resolved after combination therapy with oral prednisolone and cyclosporine. 2. Case report A 33-year-old man presented with complaint of diminution of vision in his right eye which was associated with severe swelling and drooping of the upper lid (Fig. 1A) for 5 days. Past history revealed that he had episodic limb weakness for the last 2 years. Although his limb weakness resolved completely within 5 days of the recent episode, he noticed redness and swelling of the upper
∗ Corresponding author at: 123B Gautam Nagar, New Delhi 110049, India. Tel.: +91 9868918469. E-mail address: [email protected] (N.R. Bamrolia).
lid of right eye associated with diminution of vision for which he presented to us. On physical examination, there was no neurological deficit but he had dryness of mouth. On ocular examination, his visual acuity was counting fingers close to face in the right eye, and 6/6 in the left. There was mechanical ptosis due to swelling of the upper lid of the right eye. Slit lamp evaluation of the right eye showed a 3 mm × 4 mm size epithelial defect in the centre of cornea without any infiltrate (Fig. 2A), congestion of the bulbar conjunctiva (Fig. 2B) and a thick pseudomembrane on the upper palpebral conjunctiva which was adherent to adjacent bulbar conjunctiva. It was difficult to separate the pseudomembrane from the underlying conjunctiva (Fig. 1B). There was absence of anterior chamber inflammation and hypopyon, and the fundus was not visible in the right eye. The ocular examination was normal in the left eye. He was prescribed topical carboxy methyl cellulose 0.5% lubricant drops and ointment for night application and referred to a neurologist for further evaluation of his systemic disease. Haematological, immunological and radiological investigations were performed. Haematological investigations were normal except for ESR which was raised. (haemogram – 12.5 gm/dl, differential count: P – 64%, L – 32%, E – 0, M – 0, B – 0, platelet count – 2 lakh/mm3 , ESR – 42 mm/1st hour). Blood urea, creatinine, bilirubin, aminotransferases, alkaline phosphatase, electrolytes, lipid profile, fasting and postprandial blood sugar and 24 h urine vanillylmandelic acid were normal. On immunological tests for connective tissue disorder, the SS-B/La antibody titre was raised (10.33 U/ml) but the SS-A/Ro was
1367-0484/$ – see front matter © 2011 British Contact Lens Association. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.clae.2011.10.002
86
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Fig. 1. (A) Clinical photograph of the patient at presentation revealing swelling and inflammation of upper lid of the right eye. (B) Clinical photograph of the upper lid of right eye of the patient revealing thick pseudomembrane on the palpebral conjunctiva. (C) Resolution of swelling and inflammation of upper lid following addition of cyclosporine. (D) Resolution of pseudomembrane on palpebral conjunctiva following addition of cyclosporine.
normal (0.8 U/mL) while the rheumatoid factor (<10 IU/mL; normal <10 IU/mL), anti nuclear antibody, anti DsDNA(4.0 IU/mL; normal <20 IU/mL), anti neutrophil cytoplasmic antibody titres (pANCA and cANCA) were negative. Radiological investigation including chest radiograph and contrast enhanced computed tomography of the abdomen were normal. As the patient did not have the typical features of other connective tissue disorders and fulfilled four of the six criteria of the revised international classification criteria for Sjögren’s syndrome (presence of signs and symptoms of dry eye and dry mouth along with positivity of highly specific marker autoantibodies-SSB/La), a diagnosis of primary Sjogren’s syndrome with intermitant pure motor quadriparesis was made. Subsequently, oral prednisolone 60 mg once daily was started by the neurologist and he was instructed to come for biweekly follow up visits. At 4 weeks following his initial presentation, his ocular condition had progressively deteriorated. Slit lamp evaluation revealed a large corneal epithelial defect of size 7 mm × 8 mm, with stromal haze. The anterior chamber structures could not be visualized and large patch of conjunctival-scleral necrosis of 14 mm × 7 mm in size, extending 1 mm from the limbus on the superotemporal bulbar conjunctiva was noted (Fig. 2C and D). In view of the progressive worsening of the ocular surface, oral cyclosporine 50 mg thrice daily was added, after consultation with the neurologist. After 1 week of oral cyclosporine therapy, although the conjunctival-scleral necrosis and pseudomembrane started showing signs of resolution, the corneal epithelial defect persisted (Figs. 1C and D, 2E and F). In view of presence of the non-healing corneal epithelial defect and progressive onset of cicatrial entropion, a paramedian tarsorrhaphy was performed with minimal scrapping of the lid margin due to the risk of aggravating the inflammation and necrotic process further. The tissue scrapings were insufficient for histopathological evaluation. After 3 months of immunosuppressive therapy, the visual acuity improved to 6/36, the conjunctival-scleral necrosis had resolved and the corneal epithelium had healed (Fig. 2G and H). Dose of prednisolone was tapered to 10 mg once daily after 1 month of addition of cyclosporine. Immunosuppressive therapy was discontinued after completion of 6 months of treatment. The ocular and systemic condition is presently clinically stable after stopping of immunosuppressive therapy for 6 months. 3. Discussion Sjogren’s syndrome is a multisystem disease that often presents as a dry eye and dry mouth complex due to involvement of
the lacrimal and salivary gland [1]. Up to one third of patients of Sjogren’s syndrome present with extraglandular manifestation involving lung, liver, kidney, blood vessels, and the nervous system [2]. In some patients neurological manifestations may precede ophthalmic manifestation [3]. Due to variability in the presentation of the disease, the diagnosis is often delayed. Similarly, in our case the diagnosis was delayed by 3 years. Our patient presented with neurological manifestation as intermitant quadriparesis preceding an atypical ocular presentation in the form of lid swelling causing mechanical ptosis associated with signs of severe ocular surface inflammation including pseudomembraneous conjunctivitis and conjunctival-scleral necrosis. The diagnosis of Sjogren’s syndrome was made after excluding other connective tissue disorders (wegner’s granulomatosis, systemic lupus erythematosis, rheumatoid arthritis) which may also be associated with ocular manifestations. Wegner’s granulomatosis which also presents as necrotising keratoscleritis was clinically excluded in our patient due to the absence of pulmonary, renal and sinus involvement, along with absence of cANCA and pANCA positivity [4]. Moreover this disease is very rare in India [5]. Conjunctivalscleral necrosis has been described in systemic vasculitis such as Wegner’s granulomatosis, however to the best of our knowledge pseudomembraneous conjunctivitis with conjunctival-scleral necrosis as the presenting feature of Sjogren’s syndrome has not been described previously. The extraglandular features of Sjogren’s syndrome are often managed with systemic glucocorticoids and immunosuppressive drugs [6]. Due to progression of conjunctival-scleral necrosis despite initial treatment with oral prednisolone alone, oral cyclosporine was added. This combination therapy along with tarsorrhaphy helped us to manage the case satisfactorily. Cyclosporine when combined with corticosteroid has been reported to have better therapeutic efficacy as well as exert steroid sparing effect in various ocular and non-ocular conditions [7–9]. Interestingly, the beneficial effect of the combination of oral cyclosporine and prednisolone for treatment of ocular complications in Sjogren’s syndrome, has been reported only in few cases [10]. Thus patients presenting with unexplained pseudomembraneous conjunctivitis, conjunctival-scleral necrosis and neurological symptoms, should be evaluated with a high index of suspicion for Sjogren’s syndrome with or without systemic vasculitis. Such patients presenting with severe forms of ocular surface inflammation, may benefit from the combination of oral prednisolone and cyclosporine. Nevertheless, the important role of tarsorrhaphy in
N.R. Bamrolia et al. / Contact Lens & Anterior Eye 35 (2012) 85–88
87
Fig. 2. Composite sequential photographs of the right eye of the patient. (A and B) At presentation: (A) A 3 mm × 5 mm corneal epithelial defect (B). Absence of necrosis of bulbar conjunctiva. (C and D) At 4 weeks following presentation: (C) A large corneal epithelial defect of size 7 mm × 8 mm, with stromal haze and non visualization of the anterior chamber structures. (D) A large patch of conjunctival-scleral necrosis 14 mm × 7 mm dimension, 1 mm from the limbus on the superotemporal bulbar conjunctiva was noted. (E and F) After 1 week of oral immunosuppressive therapy: (E) The corneal epithelial defect persisted. (F) The conjunctival-scleral necrosis had resolved markedly. (G and H) At 12 weeks following presentation: (G) Corneal epithelium had healed. (H) Conjunctival-scleral necrosis had resolved completely.
maintaining the tear film and thus facilitating healing of the corneal epithelium should also not be ignored. Conflict of interest None. References [1] Ramos-Casals M, Tzioufas AG, Stone JH, Sisó A, Bosch X. Treatment of primary Sjögren syndrome: a systematic review. JAMA 2010;304(July (4)):452–60.
[2] Shan SJ, Wu EI, Akpek EK. Sterile corneal melt after descemet stripping endothelial keratoplasty in patients with previously undiagnosed Sjogren syndrome. Arch Ophthalmol 2009;127(February (2)):219–20. [3] Alhomoud IA, Bohlega SA, Alkawi MZ, Alsemari AM, Omer SM, Alsenani FM. Primary Sjogren’s syndrome with central nervous system involvement. Saudi Med J 2009;30(August (8)):1067–72. [4] Chua J, Lim L. Systemic Wegener’s granulomatosis with severe orbito-ocular involvement. Singapore Med J 2008;49(October (10)):e259–62. [5] Biswas J, Babu K, Gopal L, Krishnakumar S, Suresh S, Ramakrishnan S. Ocular manifestations of Wegener’s granulomatosis. Analysis of nine cases. Indian J Ophthalmol 2003;51(September (3)):217–23. [6] Wakamatsu TH, Sato EA, Matsumoto Y, Ibrahim OM, Dogru M, Kaido M, Ishida R, Tsubota K. Conjunctival in vivo confocal scanning laser microscopy in patients with Sjögren syndrome. Invest Ophthalmol Vis Sci 2010;51(January (1)):144–50 [Epub 2009 August 20].
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[7] Panaccione R, Rutgeerts P, Sandborn WJ, Feagan B, Schreiber S, Ghosh S. Review article: treatment algorithms to maximize remission and minimize corticosteroid dependence in patients with inflammatory bowel disease. Aliment Pharmacol Ther 2008;28(September (6)):674–88. [8] Lam DS, Wong AK, Tham CC, Leung AT. The use of combined intravenous pulse methylprednisolone and oral cyclosporin A in the treatment of corneal graft rejection: a preliminary study. Eye (Lond) 1998;12(4):615–8.
[9] Niaudet P, Fuchshuber A, Gagnadoux MF, Habib R, Broyer M. Cyclosporine in the therapy of steroid-resistant idiopathic nephrotic syndrome. Kidney Int Suppl 1997;58:S85–90. [10] Cordero-Coma M, Anzaar F, Sobrin L, Foster CS. Systemic immunomodulatory therapy in severe dry eye secondary to inflammation. Ocul Immunol Inflamm 2007;15(March–April (2)):99–104.